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先天性巨细胞病毒感染导致噬血细胞性淋巴组织细胞增生症。

Haemophagocytic lymphohistiocytosis secondary to intrauterine cytomegalovirus infection.

机构信息

Universidade CEUMA, São Luís, Maranhão, Brazil.

Hospital da Criança Dr. Odorico de Amaral Matos, São Luís, Maranhão, Brazil.

出版信息

Rev Inst Med Trop Sao Paulo. 2021 Mar 1;63:e15. doi: 10.1590/S1678-9946202163015. eCollection 2021.

Abstract

Congenital cytomegalovirus infection causes lethal diseases with neurological, visual, auditory and systemic injuries, including the hemophagocytic syndrome. Hemophagocytic lymphohistiocytosis (HLH) can be caused by primary hereditary immunological defects, as well as several infectious triggering factors, such as viruses, bacteria and fungus, among them the cytomegalovirus (CMV). Here we present the case report of a male newborn male, delivered by cesarean at term (gestation age of 39 weeks), weighing 3,250 g, with suffusion skin lesions spread throughout the body, anemia, generalized edema, hepatosplenomegaly, thrombocytopenia associated with grunts and difficulty breathing, treated with ganciclovir after receiving the diagnosis of congenital CMV infection. After a few days of hospitalization, the patient presented with high fever, persistent hepatosplenomegaly and pancytopenia, in addition to elevated ferritin and triglycerides, receiving the diagnosis of HLH treated with immunosuppressive therapy, corticosteroids and intravenous human immunoglobulin. The present case report highlights the importance for health professionals to carry out the investigation of congenital diseases, especially in developing countries, as well as their complications, such as HLH.

摘要

先天性巨细胞病毒感染可导致具有神经、视觉、听觉和全身损伤的致死性疾病,包括噬血细胞综合征。噬血细胞性淋巴组织细胞增生症(HLH)可由原发性遗传性免疫缺陷引起,也可由多种感染性触发因素引起,如病毒、细菌和真菌,其中包括巨细胞病毒(CMV)。本文报道了一例男性新生儿病例,经剖宫产足月分娩(胎龄 39 周),体重 3250g,全身弥漫性皮肤淤斑、贫血、全身性水肿、肝脾肿大、伴有咕噜声和呼吸困难的血小板减少,诊断为先天性 CMV 感染后接受更昔洛韦治疗。住院几天后,患者出现高热、持续肝脾肿大和全血细胞减少,铁蛋白和甘油三酯升高,诊断为噬血细胞性淋巴组织细胞增生症,接受免疫抑制治疗、皮质类固醇和静脉注射人免疫球蛋白治疗。本病例报告强调了卫生专业人员开展先天性疾病调查的重要性,尤其是在发展中国家,以及这些疾病的并发症,如 HLH。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/607c/7924979/666a4595c073/1678-9946-rimtsp-63-S1678-9946202163015-gf01.jpg

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